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Huntington Disease

  • Andrea E. Cavanna
Chapter

Abstract

Huntington disease is a rare neurodegenerative movement disorder characterised by abrupt, irregular, and purposeless ‘dance-like’ movements. Huntington disease is a genetic condition inherited in an autosomal dominant fashion, with expansion of the trinucleotide CAG on the short arm of chromosome 4 identified as single genetic change. At a population level, age at onset of motor manifestations is inversely proportional to CAG repeat length, with higher numbers of repeats correlating with earlier development of symptoms. Higher CAG lengths (for instance, 55 and over) can lead to a juvenile form of Huntington disease (‘Westphal variant’), with more pronounced rigidity, akinesia, and cognitive changes, as well as psychiatric symptoms including depression, anxiety, impulsivity, and aggression. Overall, behavioural symptoms are common in Huntington disease. Specific changes in personality and behaviour are often more disabling than the motor features. Affective symptoms occur in about half of the patients and anxiety in about 20%. Irritability is also highly prevalent and usually occurs in the wider context of psychorigidity and deficits in social cognition. Dementia develops in all patients with Huntington disease later in the illness, whereas alterations in social cognition can occur in earlier stages of the illness.

Keywords

Chorea Huntington disease Movement disorder Social cognition Westphal variant 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Andrea E. Cavanna
    • 1
  1. 1.Dept of NeuropsychiatryUniversity of BirminghamBirminghamUnited Kingdom

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